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1 Medical Retina Service, Moorfields Eye Hospital, City Road, London EC1V 2PD, UK
2 Department of Ophthalmology, New York University School of Medicine, New York, NY, USA
3 Institute of Ophthalmology 11–43 Bath Street, London EC1V 9EL, UK
4 Medical Retina Service, Moorfields Eye Hospital, City Road London EC1V 2PD, UK
Correspondence to:
MrC Pavesio
Moorfields Eye Hospital, City Road, London EC1V 2PD, UK; cpavesio@aol.com
Accepted for publication 16 November 2004
Keywords: chronic lymphocytic leukaemia; scleritis
Acute leukaemia is known to affect the eye in a wide variety of ways and detailed postmortem examination will often reveal subclinical involvement. Ocular involvement by chronic leukaemia is much less common.1 We describe the clinical presentation, evaluation, and response to therapy of what is to our knowledge the first case of clinically significant scleral infiltration by chronic lymphocytic leukaemia mimicking scleritis.
Case report
An 87 year old man presented with a 2 week history of right eye redness, pain, and decreased vision. He complained of horizontal diplopia for 2 years, which had been corrected with prisms, but never investigated. Past medical history was notable only for chronic lymphocytic leukaemia (CLL), which was first diagnosed 17 years before presentation. He was treated intermittently with chlorambucil, but this was stopped as his white cell count had been stable at 30x109/l with minimal cervical and axillary lymphadenopathy. Vision was 6/18 right eye and 6/9 left eye. Intraocular pressure (IOP) was 18 mm Hg in each eye. His vision was reduced by bilateral early cataracts and his right eye showed inferior scleral injection (fig 1A), a fibrinous anterior uveitis, and scattered posterior synechiae.
Figure 1 (A) Right eye showing diffuse injection of the inferior sclera. (B) Computed tomography (CT) scan of orbit showing a mass that was confluent with the sclera.
The patient was treated with topical corticosteroids and cycloplegics, but over the 3 weeks following presentation his vision dropped to 6/60 right eye because of choroidal effusions. He had restricted movement of the right eye, proptosis, and shallowing of the anterior chamber with an increase in IOP to 26 mm Hg. Ultrasound revealed thickening of the inferior sclera. A CT scan of the right orbit revealed a mass in the inferior fornix that was attached to the sclera (fig 1B). Excision biopsy revealed patchy infiltration by foci of densely packed small lymphocytic cells, mainly around small blood vessels (fig 2A). Immunostaining showed a predominance of weakly stained CD20 positive B lymphocytes with scattered CD3 positive T cells consistent with CLL. Haematological analysis revealed a total white cell count of 49.1x109/l. He received orbital radiotherapy 27.5 Gy in 11 fractions using 6 MV x rays, completing the course 5 months after presentation. The inflammation, choroidal effusions, and orbital mass regressed completely but he was left with thinning of the inferior sclera (fig 2B). He was last seen 2 years after presentation with vision of 6/60 right eye but no evidence of active ocular involvement from his CLL. He refused cataract surgery.
Figure 2 (A) Histopathology of scleral mass showing foci of densely packed small lymphocytic cells, mainly around small blood vessels (haematoxylin and eosin stain, original magnification x200). (B) Right eye following therapy showing thinning of the inferior sclera.
Comment
This patient presented with what at first appeared to be a scleritis with a secondary fibrinous anterior uveitis. He was 87 years old, which is an unusual age for a first presentation of either scleritis or anterior uveitis. His history of chronic diplopia and past medical history of CLL suggested a possible aetiology for these findings. It is uncommon for CLL to affect the eye in a clinically significant way2 although postmortem examination has shown relatively frequent subclinical involvement in between 30% and 90% of patients.3 Despite this, CLL has been described causing uveitis, glaucoma, iris infiltration,4 retinal detachments, conjunctival vascular changes, optic neuropathy, and orbital involvement as well as the more typical retinal haemorrhagic changes.
Episcleral and scleral infiltration is recognised in acute leukaemia and has been seen at necropsy in chronic leukaemia in 14% of cases in one series but this is rarely symptomatic.3
In our patient the response to radiotherapy was encouraging and although his final acuity was not improved this was due in part to the progression of his cataracts. In summary, this is the first case we are aware of in which CLL had infiltrated the sclera of an eye to a sufficient degree that it simulated scleritis. The scleral mass regressed completely following radiotherapy although vision did not recover.
References
Allen RA, Straatsma BR. Ocular involvement in leukemia and allied disorders. Arch Ophthalmol 1961;66:490–508.
Buchan J, McKibbin M, Burton T. The prevalence of ocular disease in chronic lymphocytic leukaemia. Eye 2003;17:27–30.
Kincaid MC, Green WR. Ocular and orbital involvement in leukemia. Surv Ophthalmol 1983;27:211–32.
Martin B . Infiltration of the iris in chronic lymphatic leukaemia. Br J Ophthalmol 1968;52:781–5.